在线日韩日本国产亚洲丨少妇伦子伦情品无吗丨欧美性猛交xxxx免费看蜜桃丨精品人妻系列无码一区二区三区丨亚洲精品无码不卡在线播放

Your Good Partner in Biology Research

  1. 首頁(yè)
  2. 蛋白
  3. 重組蛋白
  4. Recombinant Human Ceroid-lipofuscinosis neuronal protein 6 (CLN6), partial

Recombinant Human Ceroid-lipofuscinosis neuronal protein 6 (CLN6), partial

  • 中文名稱：
    Recombinant Human Ceroid-lipofuscinosis neuronal protein 6(CLN6),partial
  • 貨號(hào)：
    CSB-YP882125HU1
  • 規(guī)格：
  • 來(lái)源：
    Yeast
  • 其他：
  • 中文名稱：
    Recombinant Human Ceroid-lipofuscinosis neuronal protein 6(CLN6),partial
  • 貨號(hào)：
    CSB-EP882125HU1
  • 規(guī)格：
  • 來(lái)源：
    E.coli
  • 其他：
  • 中文名稱：
    Recombinant Human Ceroid-lipofuscinosis neuronal protein 6(CLN6),partial
  • 貨號(hào)：
    CSB-EP882125HU1-B
  • 規(guī)格：
  • 來(lái)源：
    E.coli
  • 共軛：
    Avi-tag Biotinylated

    E. coli biotin ligase (BirA) is highly specific in covalently attaching biotin to the 15 amino acid AviTag peptide. This recombinant protein was biotinylated in vivo by AviTag-BirA technology, which method is BriA catalyzes amide linkage between the biotin and the specific lysine of the AviTag.

  • 其他：
  • 中文名稱：
    Recombinant Human Ceroid-lipofuscinosis neuronal protein 6(CLN6),partial
  • 貨號(hào)：
    CSB-BP882125HU1
  • 規(guī)格：
  • 來(lái)源：
    Baculovirus
  • 其他：
  • 中文名稱：
    Recombinant Human Ceroid-lipofuscinosis neuronal protein 6(CLN6),partial
  • 貨號(hào)：
    CSB-MP882125HU1
  • 規(guī)格：
  • 來(lái)源：
    Mammalian cell
  • 其他：

產(chǎn)品詳情

  • 純度：
    >85% (SDS-PAGE)
  • 基因名：
    CLN6
  • Uniprot No.：
  • 別名：
    CLN6; Ceroid-lipofuscinosis neuronal protein 6; Protein CLN6
  • 種屬：
    Homo sapiens (Human)
  • 蛋白長(zhǎng)度：
    Partial
  • 蛋白標(biāo)簽：
    Tag?type?will?be?determined?during?the?manufacturing?process.
    The tag type will be determined during production process. If you have specified tag type, please tell us and we will develop the specified tag preferentially.
  • 產(chǎn)品提供形式：
    Lyophilized powder
    Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
  • 復(fù)溶：
    We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.
  • 儲(chǔ)存條件：
    Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
  • 保質(zhì)期：
    The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
    Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
  • 貨期：
    Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
    Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
  • 注意事項(xiàng)：
    Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
  • Datasheet ：
    Please contact us to get it.

產(chǎn)品評(píng)價(jià)

靶點(diǎn)詳情

  • 基因功能參考文獻(xiàn)：
    1. The CLN6 is not only a molecular entity of the anti-aggregate activity conferred by the ER manipulation using TMalphaBC, but also serves as a potential target of therapeutic interventions. PMID: 28476624
    2. describe the spectrum of clinical and neurophysiologic features associated with mutations of CLN6. PMID: 26115733
    3. study demonstrates the central role of the metal transporter, Zip7, in the aberrant biometal metabolism of CLN6 variants of Neuronal ceroid lipofuscinoses. PMID: 24581221
    4. The study describes the first report in the North of Morocco of the CLN6 p.I154del mutation in 3 patients belonging to a large consanguineous family. PMID: 23180398
    5. our results add CLN6 to the genetic mutations causing teenage-onset progressive myoclonus epilepsy PMID: 22883287
    6. CLN6 and CLN3 mutations trigger distinct processes that converge on a shared pathway, which is responsible for proper subunit c protein turnover and neuronal cell survival. PMID: 21359198
    7. Sequencing of CLN6 will provide a simple diagnostic strategy in this disorder, in which definitive identification usually requires invasive biopsy. PMID: 21549341
    8. Expression studies of three mutations found in CLN6 patients predicted to affect transmembrane domain 3, cytoplasmic loop 2 or result in a truncated membrane protein respectively, is reported. PMID: 20020536
    9. gene mutated in variant late-infantile neuronal ceroid lipofuscinosis (CLN6) and in nclf mutant mice encodes a novel predicted transmembrane protein PMID: 11727201
    10. novel approximately 36-kD CLN6-gene product augments an intriguing set of unrelated membrane-spanning proteins, whose deficiency causes neuronal ceroid lipofuscinosis in mouse and man PMID: 11791207
    11. Eight novel mutations identified in CLN6 in 26 families with late infantile neuronal ceroid lipofuscinosis. PMID: 12815591
    12. ER-resident CLN6 protein lead to lysosomal dysfunctions, which may result in lysosomal accumulation of storage material PMID: 15010453
    13. CLN6 is an ER resident protein, the activity of which, despite this location, must contribute to lysosomal function. PMID: 15265688
    14. These data indicate that CLN6 mutations, in addition to those of CLN8, should be considered a diagnostic alternative in Turkish variant late-infantile neuronal ceroid lipofuscinosis patients. PMID: 15996215
    15. Cholesterol accumulation in lysosomes suggests a homeostasis block as a result of CLN6p deficiency, while dysfunctional endosomal/lysosomal vesicles may act as one of the triggers for apoptosis and cell death. PMID: 16857350
    16. CLN6 maintains its endoplasmic reticulum localization by expressing retention signals present in both the N-terminal cytosolic domain and in the carboxy-proximal transmembrane domains 6 and 7. PMID: 17453415
    17. knockdown of SEL1L [sel-1 suppressor of lin-12-like (Caenorhabditis elegans)], a member of an E3 ubiquitin ligase complex involved in ER protein extraction, rescued significant amounts of Cln6(G123D) and Cln6(M241T) polypeptides. PMID: 18811591
    18. 11 mutations in patients with neuronal ceroid lipofuscinoses, eight of which are novel, were detected in CLN6, all predicting a direct impairing of the putative gene function. PMID: 19135028
    19. three families with CLN6-associated variant late infantile neuronal ceroid lipofuscinosis from Saudi Arabia are described; one had a novel mutation in the CLN6 gene PMID: 19520283

    顯示更多

    收起更多

  • 相關(guān)疾病：
    Ceroid lipofuscinosis, neuronal, 6 (CLN6); Ceroid lipofuscinosis, neuronal, 4A (CLN4A)
  • 亞細(xì)胞定位：
    Endoplasmic reticulum membrane; Multi-pass membrane protein. Endoplasmic reticulum.
  • 數(shù)據(jù)庫(kù)鏈接：

    HGNC: 2077

    OMIM: 204300

    KEGG: hsa:54982

    STRING: 9606.ENSP00000249806

    UniGene: Hs.584921



主站蜘蛛池模板： 做性久久久久久| 欧美巨大xxxx做受中文字幕| 久久久精品456亚洲影院| 图片区小说区亚洲欧美自拍| 好男人在线社区www在线影院| 无码毛片内射白浆视频| 熟女体下毛毛黑森林| 国产欧美日韩专区发布| 无翼乌工口全彩肉肉无遮挡18| 在线观看的av免费网站| 久久精品一本到东京热| 国产精品激情| 搡老熟女老女人一区二区| 久热这里只有精品视频6| 亚洲狠狠色丁香婷婷综合| 欧美性猛交xxxxx按摩欧美| 亲子乱子伦xxxx视频| 国产人与zoxxxx另类| 夜夜未满十八勿进的爽爽影院| 亚洲一卡2卡3卡4卡 精品| 免费国产成人高清在线视频 | 国产台湾无码av片在线观看| 漂亮人妻被中出中文字幕| 我的公把我弄高潮了视频| 99久久99久久久精品齐齐综合色圆| 成年网站未满十八禁视频天堂| 狠狠亚洲狠狠欧洲2019| 国产精品久久久久久久久久久免费看| yw尤物av无码国产在线观看| 国产三级不卡在线观看视频| 国产suv精二区| 亚洲色成人www永久在线观看| 巨胸不知火舞露双奶头无遮挡| 高h猛烈失禁潮喷无码视频| 无码天堂亚洲国产av麻豆| 女人19水真多免费毛片| 亚洲熟妇无码爱v在线观看| 亚洲伊人色欲综合网| 水蜜桃精品一二三| 中文字字幕在线精品乱码| 97精品人人妻人人|